2017
DOI: 10.1038/nrd.2017.170
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Abstract: Idiopathic pulmonary fibrosis (IPF) is a fatal age-associated disease that is characterized by progressive and irreversible scarring of the lung. The pathogenesis of IPF is not completely understood and current therapies are limited to those that reduce the rate of functional decline in patients with mild-to-moderate disease. In this context, new therapeutic approaches that substantially improve the survival time and quality of life of these patients are urgently needed. Our incomplete understanding of the pat… Show more

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Cited by 264 publications
(195 citation statements)
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“…However, there is no effective therapeutic strategy for this devastating lung disease. Although pirfenidone and nintedanib have been approved by the Food and Drug Administration , they only slow down the decline of lung function in patients with the mild and moderate disease . So, the development of effective treatment for PF is urged.…”
Section: Introductionmentioning
confidence: 99%
“…However, there is no effective therapeutic strategy for this devastating lung disease. Although pirfenidone and nintedanib have been approved by the Food and Drug Administration , they only slow down the decline of lung function in patients with the mild and moderate disease . So, the development of effective treatment for PF is urged.…”
Section: Introductionmentioning
confidence: 99%
“…Pulmonary fibrosis manifests in individuals of ages 55 and older, suggesting that IPF is a disease associated with aging 28,29 . Redox mechanisms have been implicated to play a role in age-related pulmonary fibrosis 6 .…”
Section: Textmentioning
confidence: 99%
“…Pulmonary fibrosis is an unrelenting progressive disease, associated with enhanced epithelial cell death, impaired epithelial progenitor function and consequently failure to restore normal epithelial barriers 22,29,31 . These processes are believed to stimulate an overpopulation of activated myofibroblasts and excessive deposition of ECM 32 .…”
Section: Textmentioning
confidence: 99%
“…To date, only a very limited number of drugs are available to target lung fibrosis. Therefore, further studies with a special focus on the distinct role of the AKAP family members such as ezrin and AKAP13 will identify new therapeutic targets and hence novel drugs in the treatment of lung fibrosis (Gourdie, Dimmeler, & Kohl, 2016;Kalluri, 2016;Mora, Rojas, Pardo, & Selman, 2017).…”
Section: Outlook and Future Perspectivesmentioning
confidence: 99%